Risk factors for morbidity and mortality in Ebstein's anomaly: a registry-based study of 398 patients

To investigate the long-term outcomes and risk factors for morbidity and mortality in patients with Ebstein's anomaly, including the effects of type and timing of valve surgery.

For this retrospective, record-based study, all patients with Ebstein's anomaly enrolled in the German National Register for Congenital Heart Defects up to June 2021 were eligible for inclusion.

Non-surgical patients (n = 194/49% of 398 patients) had less tricuspid valve regurgitation (p < 0.001) and heart failure symptoms (p < 0.001) than surgical patients (n = 204/51%). Postoperative survival at 10, 20, and 30 years was 97%, 93%, and 80%. Eighty-one (40%) patients underwent multiple surgeries. Re-operation rates were lowest in patients with first valve surgery during adolescence (p = 0.0076). Postoperative NYHA class > I was more frequent with surgery delayed to older age (p < 0.001). Initial corrective surgery was complicated by complete atrioventricular block (CAVB) in 17 (9%) of patients. CAVB was more likely with older age (p = 0.001), and tricuspid valve replacement compared to reconstruction (p = 0.029). CAVB was associated with all-cause death (p < 0.001). Cone reconstruction reduced the risk of CAVB (p = 0.008) and tricuspid valve regurgitation (p < 0.001) compared to monocusp reconstruction.

This registry-based study of Ebstein's anomaly corroborates good surgical long-term results, while re-operation rates remain high. Patients operated before adolescence were at the highest risk of re-operation, while older age at the time of the first surgery increased the risk of CAVB. The cone reconstruction was associated with improved tricuspid valve function and a lower risk of CAVB compared to monocusp reconstructions. Choosing an optimal time window for surgery and use of the cone reconstruction may therefore further improve outcomes.